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Fuchs' Dystrophy

Definition


Disease: Fuchs' Dystrophy Fuchs' Dystrophy
Category: Eye diseases
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Disease Definition:

In Fuchs' dystrophy, the cornea, the transparent front surface of the eye, is affected. This is a rare condition, which usually occurs as an inherited disorder, the cause of which is still not known.

 

Usually, excess fluid is prevented from accumulating in the cornea by the cells that line the back surface (endothelium) of the cornea. This helps maintain the cornea its transparency. However, those endotherlial cells slowly deteriorate and die off in Fuchs' dystrophy, resulting in the building up of fluid in the cornea. Because of this, swelling, pain, cloudy vision and loss of corneal transparency could result.

 

A variety of vision problems could be caused by Fuchs' dystrophy, which could eventually lead to blindness. Fuchs' dystrophy is considered one type of corneal dystrophy.
 

Work Group:


Prepared by: Scientific Section

Symptoms, Causes

Symptoms:

Early signs and symptoms of Fuchs' dystrophy may be seen in people who are in their 30s and 40s. However, most people don't experience any signs and symptoms until they're in their 50s and 60s. Usually affecting both eyes, the signs and symptoms of Fuchs' dystrophy include:

 

  • Blindness
  • Sensitivity to light
  • Difficulty seeing at night
  • Astigmatism, which is when someone sees halos around lights
  • Changes in vision, visual impairment and distorted vision
  • Blurred vision on awakening, which could gradually clear up as the day progresses
  • A cloudy or hazy appearance of the cornea
  • Tiny but painful blisters, called epithelial blisters, which occur on the surface of the cornea. The excess fluid within the cornea is the cause of this.

 

A person should see an ophthalmologist or a potometrist (an eye doctor) in case he/she experiences some of these symptoms, especially if they get worse over time. A person should also call an ophthalmologist for an urgent appointment in case the signs and symptoms develop suddenly. Prompt treatment is required for many conditions that cause the same signs and symptoms as fuchs' dystrophy.
 

Causes:

The cause of Fuchs' dystrophy is not known in some people. However, for most people it is inherited as a condition called autosomal dominant familial, which is when about half of an affected person's children also have the disease. But the signs and symptoms that relatives experience usually vary. A parent, for example, who has a mild case and a few symptoms, could have a child with a severe case and numerous problems of vision.
 

Complications

Complications:

None

Treatments:

In order to improve comfort or to stop the signs and symptoms of Fuchs' dystrophy from getting worse, a person may be suggested following methods and procedures, such as:

 

  • Wearing soft contact lenses in order to reduce discomfort and improve vision
  • Using eyedrops or ointments in order to reduce the amount of fluid in the cornea
  • Using a hair dryer and holding it at arm's length. Excess fluid in the cornea is evaporated and the blisters are dried out when direct warm, not hot, air crosses the face two or three times a day.
  • Receiving a corneal transplant, also called a keratoplasty. In this procedure, the damaged cornea tissue is replaced with healthy tissue from a donor. Many types of corneal procedures are available. While some procedures replace the entire cornea, others replace only a few thin layers of the cornea. In the case of Fuchs' dystrophy, the procedure that is commonly performed is called a posterior lamellar keratoplasty, or an endotherlial keratoplasty, in which only the deep layers of the cornea are replaced, including the endothelium.

 

Corneal transplants have high success rates and are quite common. However, until a donor tissue becomes available, the patient may have to wait a long time. Some people may develop problems following transplants. In case the transplant recipient's body attempts to reject the new corneal tissue, it may result in worsening vision, redness and increased sensitivity. Topical eye medications could manage these rejections most of the time. However, the person's eyes may feel uncomfortable after a corneal transplant and in order to help them heal, the patient will have to use eyedrops for several months. Usually, people who've had a successful transplant for Fuchs' dystrophy will not experience any symptoms for at least ten years.
 

Prognosis:

Not available

Expert's opinion

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